A Case Report of Double Valve Replacement for Mucopolysaccharosis with Chest Pain Attack and Severe Heart Failure

VernacularTitle:胸痛発作と重症心不全を伴ったムコ多糖症に対する２弁置換術の１例
Author: Yukihiro Katayama ; Ryuji Kunitomo ; Kentaro Takaji ; Ryusuke Suzuki ; Hisashi Sakaguchi ; Ichiro Ideta ; Michio Kawasuji
Publication Type:Journal Article
From:Japanese Journal of Cardiovascular Surgery 2005;34(4):317-320
CountryJapan
Language:Japanese
Abstract: We report a successfully treated case of double valve replacement for mucopolysaccharosis in a 27-year-old woman. Mucopolysaccharosis had been suspected since she was aged 11. Symptoms of heart failure and chest pain suggested valvular disease and she was referred to us. Echocardiography, aortography and cardioangiography showed aortic regurgitation (grade IV/IV) and mitral regurgitation (grade III/IV). She received double valve replacement and was discharged on the 38th postoperative day with symptom improvement. Although urinalysis was positive for heparan-sulfate, this case could not be diagnosed definitively as mucopolysaccharosis due to normal lymphocytic enzyme-activity. However, large amounts of mucopolysaccharoid deposits were present in her removed aortic and mitral valve leaflets, and her clinical picture corresponded with mucopolysaccharosis. Thus, it was considered that her ultimate diagnosis was combined cardiac valvular disease due to mucopolysaccharosis.